Annals of Indian Academy of Neurology
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CASE REPORT
Year : 2009  |  Volume : 12  |  Issue : 1  |  Page : 48-51

Heidenhain variant of Creutzfeldt-Jakob disease: An autopsy study from India


1 Department of Medicine, MS Ramaiah Medical College and Hospital, MSRIT Nagar, Bangalore - 560 054, India
2 Department of Neurology, MS Ramaiah Medical College and Hospital, MSRIT Nagar, Bangalore - 560 054, India
3 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore - 560 029, India

Correspondence Address:
Medha Y Rao
650, 12th Cross, MC Layout, Vijayanagar, Bangalore - 560 040
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.48856

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Prion diseases are rare, progressive and fatal neurodegenerative diseases characterized by long incubation period and short clinical course. We present a rare case of Heidenhain variant of Creutzfeldt-Jakob disease, occurring in a 55-year-old lady presenting with dementia, cortical blindness, and myoclonic jerks. She succumbed to the disease within 8 weeks of onset of symptoms. MRI revealed hyperintense signals on T2WI and fluid attenuated inversion recovery (FLAIR) images in basal ganglia and fronto-temporal and parietal cortex, sparing thalamus, striate cortex and globus pallidum. Abundant abnormal prion protein deposits (PrP sc) were detected in caudate, putamen, thalamus, cingulate and striate cortex, in comparison to frontal and parietal cortex while no deposits were found in globus pallidum. MRI changes did not correlate with degree of spongy change, gliosis or prion protein deposition. The cause for abnormal signal changes in MRI and FLAIR images remains unclear.


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